Clinical Rheumatology

, Volume 16, Issue 6, pp 570–577 | Cite as

Clinical, laboratory and radiological findings in pulmonary fibrosis with and without connective tissue disease

  • E. Renzoni
  • P. Rottoli
  • G. Coviello
  • M. G. Perari
  • M. Galeazzi
  • M. Vagliasindi


Uncertainty exists over whether to consider “lone” idiopathic pulmonary fibrosis (LIPF) and pulmonary fibrosis associated with connective tissue disorders (PFCTD) as significantly different entities. We retrospectively analysed data collected at the time of first diagnosis in 17 patients with LIPF and in 14 patients with PFCTD and compared survival in the two groups. At first evaluation, the time from onset of respiratory symptoms, spirometric volumes and the diffusing capacity for carbon monoxide were not significantly different between the two groups. However, arterial oxygen tension was significantly lower in LIPF than in PFCTD (63±3 vs 88±3 mmHg, p<0.001). The radiological profusion scores relative to the upper and middle lung fields were significantly higher in LIPF than in PFCTD (upper regions: 6.9±0.6 vs 3.4±0.6, p<0.005 - middle regions: 7.1±0.5 vs 4.8±0.7, p<0.025), whereas the scores relative to the lower fields were similar (7.4±0.4 in LIPF and 8.4±0.6 in PFCTD). Survival since onset of respiratory symptoms was significantly better in the PFCTD than in LIPF patients, with a hazard ratio of 4.16 (95% CI 1.12–15.58, p=0.034). Thus, in our series of patients, those with LIPF had a more severe disease than those with PFCTD as shown by the higher frequency of hypoxaemia, the more diffuse pulmonary involvement demonstrated by the chest X-ray and the decreased survival.

Key words

Idiopathic Pulmonary Fibrosis Connective Tissue Disease Radiology Pulmonary Function Survival 


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  1. 1.
    Crystal RG, Fulmer JD, Roberts WC, Moss ML, Line BR, Reynolds HY. Idiopathic pulmonary fibrosis: clinical, histologic, radiographic, physiologic, scintigraphic, cytologic and biochemical aspects. Ann Intern Med 1976; 85: 769–88.Google Scholar
  2. 2.
    Turner-Warwick M, Burrows B, Johnson A. Cryptogenic fibrosing alveolitis; clinical features and their influence on survival. Thorax 1980; 35: 171–80.Google Scholar
  3. 3.
    Turner-Warwick. Interstitial pulmonary fibrosis (IPF) with and without associated “collagen vascular disease”. Am Rev Resp Dis 1981; 123 (4 part 2): 73.Google Scholar
  4. 4.
    Harrison NK, Myers R, Corrin B, Soosay G, Dewar A, Black CM, du Bois RM Turner-Warwick M. Structural features of interstitial lung disease in systemic sclerosis. Am Rev Resp Dis 1991; 144: 706–13.Google Scholar
  5. 5.
    Terriff BA, Kwan SY, Chan-Yeung MM, Muller NL. Fibrosing alveolitis: chest radiography and CT as predictors of clinical and functional impairment at follow-up in 26 patients. Radiology 1992; 184: 445–9.Google Scholar
  6. 6.
    Staples CA, Muller NL, Vedal S, Abboud R, Ostrow D, Miller RR. Usual interstitial pneumonia: correlation of CT with clinical, functional, and radiologic findings. Radiology 1987; 162: 377–81.Google Scholar
  7. 7.
    Agusti C, Xaubet A, Roca J, Agusti AGN, Rodriguez-Roisin R. Interstitial pulmonary fibrosis with and without associated collagen vascular disease: results of a two year follow up. Thorax 1992; 47: 1035–40.Google Scholar
  8. 8.
    Wells AU, Cullinan P, Hansell DM, Rubens MB, Black CM, Newman-Taylor AJ, du Bois RM. Fibrosing alveolitis associated with systemic sclerosis has a better prognosis than lone cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med 1994; 149: 1583–90.Google Scholar
  9. 9.
    Owens GR, Paradis IL, Gryzan S, Medsger TA, Follansbee WP, Klein HA, Dauber JH. Role of inflammation in the lung disease of systemic sclerosis: comparison with idiopathic pulmonary fibrosis. J Lab Clin Med 1986; 107: 253–60.Google Scholar
  10. 10.
    Raghu G. Interstitial lung disease: a diagnostic approach. Are CT scan and lung biopsy indicated in every patient? Am J Respir Crit Care Med 1995; 151: 909–14.Google Scholar
  11. 11.
    Subcommittee for scleroderma. Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 1980; 23: 581–90.Google Scholar
  12. 12.
    Arnett FC, Edworthy SM, Bloch DA, Mcshane DJ, Fries JF, Cooper NS et al. The American Rheumatism Association 1987 revised criteria for the classification of rheumatoid arthritis. Arthritis Rheum 1988; 31: 315–24.Google Scholar
  13. 13.
    Bohan A, Peter JB. Polymyositis and dermatomyositis. N Engl J Med 1975; 291: 344.Google Scholar
  14. 14.
    International Labour Office. Guidelines for the use of ILO international classification of radiographs of pneumoconiosis. Rev ed. International Labour Office occupational safety and health series no. 22 (Rev 80). Geneva: International Labour Office, 1980.Google Scholar
  15. 15.
    Reynolds HY. Bronchoalveolar Lavage. Am Rev Resp Dis 1987; 135: 250–63.Google Scholar
  16. 16.
    American Thoracic Society. Clinical role of bronchoalveolar lavage in adults with pulmonary disease. Am Rev Respir Dis 1990; 142: 481–6.Google Scholar
  17. 17.
    Clayton D, M Hills Statistical models in epidemiology, Oxford University Press, Oxford 1993Google Scholar
  18. 18.
    Schneider PD, Wise RA, Hochberg MC, Wigley FM. Serial pulmonary function in systemic sclerosis. The American Journal of Medicine. 1982; 73: 385–94.Google Scholar
  19. 19.
    Greenwald GI, Tashkin DP, Gong H, Simmons M, Duann S, Furst DE, Clements P. Longitudinal changes in lung function and respiratory symptoms in progressive systemic sclerosis; a prospective study. Am J Med 1987; 83: 83–91.Google Scholar
  20. 20.
    Colp CR, Riker J, Williams MH. Serial changes in scleroderma and idiopathic interstitial lung disease. Arch Intern Med 1973; 132: 506–15.Google Scholar
  21. 21.
    Hunninghake GW, Fauci AS. Pulmonary involvement in the collagen vascular diseases. Am Rev Resp Dis 1979; 119: 471–97.Google Scholar
  22. 22.
    Johkoh T, Ikezoe J, Kohno N, Takeuchi N, Yamagami H, Tomiyama N, Kondoh H, Kido S, Arisawa J, Kozuka T. High resolution CT and pulmonary function tests in collagen vascular disease: comparison with idiopathic pulmonary fibrosis. Eur J Rad 1994; 18: 113–21.Google Scholar
  23. 23.
    Schurawitski H, Stiglbauer R, Graninger W, Herold C, Polzleitner D, Burghuber OC, Tscholakoff D. Interstitial lung disease in progressive systemic sclerosis: high resolution CT versus radiography. Radiology 1990; 176: 755–9.Google Scholar
  24. 24.
    Wells AU, Hansell DM, Corrin B, Harrison NK, Goldstraw P, Black CM, Du Bois RM. High resolution computed tomography as a predictor of lung histology in systemic sclerosis. Thorax 1992; 47: 738–42.Google Scholar
  25. 25.
    Staples CA, Muller NL, Vedal S, Abboud R, Ostrow D, Miller R. Usual Interstitial pneumonia: correlation of CT with clinical, functional and radiological findings. 1987. Radiology; 162: 377–81.Google Scholar
  26. 26.
    Grenier P, Valeyre D, Cluzel P, Braunner MW Lenoir S, Chastung C. Chronic diffuse interstitial lung disease; diagnostic value of chest radiography and high resolution CT. Radiology 1991; 179: 123–32.Google Scholar
  27. 27.
    Wells AU, Hansell DM, Cailes J, Rubens MB, Black CM, Haslam PL, du Bois RM. Bronchoalveolar lavage cellularity in fibrosing alveolitis: a comparison between lone cryptogenic fibrosing alveolitis and the fibrosing alveolitis of systemic sclerosis. Eur Resp J abstracts 1995; 8 (suppl 19): 453s.Google Scholar
  28. 28.
    Southcott AM, Jones KP, Li D, Majumdar S, Cambrey AD, Pantelidis P, Black CM, Laurent GJ, Davies BH, Jeffrey PK, Du Bois RM. Interleukin-8: differential expression in lone fibrosing alveolitis and systemic sclerosis. Am J Respir Crit Care Med 1995; 151: 1604–12.Google Scholar

Copyright information

© Clinical Rheumatology 1997

Authors and Affiliations

  • E. Renzoni
    • 3
  • P. Rottoli
    • 3
  • G. Coviello
    • 2
  • M. G. Perari
    • 3
  • M. Galeazzi
    • 1
  • M. Vagliasindi
    • 3
  1. 1.Institute of Rheumatology of the University of SienaItaly
  2. 2.Radiology Unit, Siena HospitalItaly
  3. 3.Institute of Respiratory DiseasesUniversity of Siena, Ospedale “Le Scotte”SienaItaly

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